2qll: Difference between revisions
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===Human liver glycogen phosphorylase- GL complex=== | ===Human liver glycogen phosphorylase- GL complex=== | ||
{{ABSTRACT_PUBMED_18198182}} | {{ABSTRACT_PUBMED_18198182}} | ||
==Disease== | |||
[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:[http://omim.org/entry/232700 232700]]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.<ref>PMID:9529348</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties. | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:018198182</ref><references group="xtra"/> | <ref group="xtra">PMID:018198182</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Phosphorylase]] | [[Category: Phosphorylase]] | ||
Revision as of 05:07, 25 March 2013
Human liver glycogen phosphorylase- GL complexHuman liver glycogen phosphorylase- GL complex
Template:ABSTRACT PUBMED 18198182
DiseaseDisease
[PYGL_HUMAN] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:232700]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.[1]
FunctionFunction
[PYGL_HUMAN] Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties.
About this StructureAbout this Structure
2qll is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See AlsoSee Also
ReferenceReference
- ↑ Pautsch A, Stadler N, Wissdorf O, Langkopf E, Moreth W, Streicher R. Molecular recognition of the protein phosphatase 1 glycogen targeting subunit by glycogen phosphorylase. J Biol Chem. 2008 Apr 4;283(14):8913-8. Epub 2008 Jan 15. PMID:18198182 doi:10.1074/jbc.M706612200